The Assessment and Management of Biliary Atresia in Hawai'i, 2009-2023.

Although biliary atresia (BA) is a rare neonatal disorder, it remains the leading cause of pediatric end-stage liver disease. Early diagnosis of BA and treatment with the Kasai procedure can significantly reduce the need for pediatric liver transplant. Current data suggests that performing the Kasai procedure at 30-45 days of life is associated with longer native liver survival rates and reduction of the need for liver transplant. The incidence rate of BA in the state of Hawai'i is nearly double the incidence rate in the continental US. International studies have demonstrated that screening programs for BA reduce the age at diagnosis and treatment. However, there has been no statewide analysis on the ages at diagnosis or at Kasai, nor does a statewide screening program for BA exist. The purpose of this study is to review the age of diagnosis and treatment of BA to determine if the current practice in Hawai'i is in line with the published data. A retrospective chart review of all patients diagnosed with BA at the state's primary children's hospital was performed (2009-2023) and 19 patients who underwent the Kasai procedure were identified. The mean age at diagnosis is 71.4 days (n=19) and the mean age at Kasai procedure is 72.0 days (n=19). Both the average age at diagnosis and treatment for BA in Hawai'i is significantly higher than published data suggesting best outcomes at 30-45 days of life. This review suggests that the implementation of a statewide screening program for BA in Hawai'i is warranted.

Newborn screening for biliary atresia using direct bilirubin: An implementation science study.

OBJECTIVE: Biliary atresia (BA) is a liver disease of infancy characterized by obstruction of the biliary tree. Infants with BA have the best outcomes when identified early and the Kasai portoenterostomy is performed before 45 days of life (DoL). In our hospital system, the average age at Kasai was 60 DoL. To address the problem of late presentation, we implemented a two-stage BA screening strategy utilizing direct bilirubin (DB). METHODS: New institutional policies were established that all newborns were tested at 24-48 h of life, and those with levels ≥0.5 mg/dL were followed further. The infant's primary care provider was contacted to recommend a repeat DB at 2 weeks of life. If the repeat DB was ≥1.0 mg/dL, the patient was evaluated by gastroenterology. RESULTS: Over the 16 months, 3880 infants were born and 3861 (99.5%) were screened; 53 infants (1.3%) had DB levels ≥0.5 mg/dL initially. Upon repeat testing at 2 weeks, there were three groups of infants: not retested (n = 1), retested <1.0 mg/dL (n = 40), and retested ≥1.0 mg/dL (n = 12). The average time to be seen by gastroenterology was 4.3 days or 18.3 DoL. DISCUSSION: The screening included a series of steps that needed to be implemented effectively. Screening had a net false positive rate of 0.3% (12 out of 3861) and identified causes of cholestasis other than BA. BA was excluded by 28 DoL on average. Our results can provide a template for other institutions interested in implementing a BA screening protocol in their practice.

Evaluation of Hepatic Shear Wave Elastography to Assess Liver Fibrosis in Biliary Atresia Patients and Its Correlation with Liver Histology and Surgical Outcomes: A Prospective Observational Study.

Introduction The native liver survival in biliary atresia (BA) depends on various factors, and one of the crucial factors is the rate of progression of liver fibrosis after portoenterostomy, but there is no reliable investigation to assess it. This study evaluated shear wave elastography (SWE) to detect liver fibrosis in BA patients and assess its utility during follow-up. Materials and Methods This was an observational study; SWE was done preoperatively and postoperatively at 3 and 6 months. The SWE values were analyzed to determine their correlations with preoperative liver histology as well as with postoperative SWE variation between different postoperative outcomes. Results Twenty-one patients were included in the study; the preoperative SWE values were strongly correlated with liver biopsy grading ( p < 0.001). At the 3 months postoperatively, SWE was done for 18 children: 12 in group A (patent bilioenteric drainage on hepatobiliary iminodiacetic acid scan) and 6 (nonpatent) in group B; mean SWE value was 12.8 and 17.3 kPa, respectively ( p < 0.001). Ten children from group A underwent SWE 6 months postoperatively, and the mean value was 13.23 kPa. Conclusion The SWE values correlate with liver histology grading, suggesting a reliable alternative to biopsy. Additionally, the baseline SWE values and their trend during follow-up can provide information on the disease's progression.

Comparison of Da Vinci Robotic-assisted With Open Kasai Portoenterostomy for Biliary Atresia.

BACKGROUND: Robotic-assisted Kasai portoenterostomy (RAKPE) has been used to treat biliary atresia (BA). This study aimed to compare the efficacy of RAKPE and open Kasai portoenterostomy (OKPE) for BA. METHODS: Thirty-one children with type III BA who underwent surgical treatment in two centers from January 2022 to December 2023 were retrospectively collected. According to the operative techniques, the participants were divided into the RAKPE group (13 cases) and the OKPE group (18 cases). The operative time, jaundice clearance (JC) rate, and incidence of cholangitis were analyzed. RESULTS: The operative time in the RAKPE group (204.3 ± 19.9 min) was significantly longer than that in the OKPE group (186.2 ± 22.2 min), P < 0.05. However, the blood loss (8.1 ± 2.5 ml) in the RAKPE group was significantly decreased compared with the OKPE group (13.6 ± 4.8 ml), and 15.4% patient need blood transfusion in RAKEP group was litter than that 55.6% in the OKPE group, P < 0.05. The time to oral feeding (2.8 ± 0.4 days vs. 4.3 ± 0.7 days) and the time to pass ICG-positive stools (3.6 ± 0.6 days vs. 4.7 ± 0.9 days) in the RAKPE group were significantly shorter than those in the OKPE group, P < 0.05. No significant differences were observed in the bile excretion rate, hospital stay time, and JC rate. The incidence of cholangitis in the RAKPE group was significantly lower than that in the OKPE group during short follow-up. CONCLUSION: RAKPE may be associated with lower intraoperative blood loss, decrease need for postoperative transfusions and decreased rate of postoperative cholangitis compared to OKPE. LEVEL OF EVIDENCE: III.

Morio Kasai Corrects the Uncorrectable: Hepatic Portoenterostomy for Biliary Atresia.

One of the fundamental innovations of pediatric surgery is hepatic portoenterostomy for biliary atresia, widely known as the Kasai procedure for its originator, Morio Kasai (1922-2008), of Sendai, Japan. It was the first effective operation for the "uncorrectable" form of biliary atresia, where death within months from biliary cirrhosis was certain. His radical premise was that microscopic ductules present in the fibrous tissue of the porta hepatis, when transected, drained enough bile to relieve cholestatic jaundice. His first operations from 1955 to 1961 had a jaundice-free survival rate of only 8 percent, but the cures were durable. By 1982-1986 survival reached 55 percent. Adoption of his procedure in the West came slowly. The original operation in 1955 was reported in the Japanese literature in 1959, but the first English language communication came in 1968. Surgeons disbelieved his results. Scientific chauvinism held that advances in pediatric surgery came from America and Europe; Japan had little to add. Surgeons visited Sendai to learn from Kasai, notably John Lilly and R. Peter Altman, then of Washington, DC, Jacques Valayer in Paris, and Edward Howard in London. At first, mortality rates in their hands was high. As expertise developed in the West with the technically demanding operation, mortality fell and approached those reported by Kasai. Successes in America, France, and England proved Kasai's operation as effective and one of signal achievements in pediatric surgery.

Human Liver Organoids to Predict the Outcome of Kasai Portoenterostomy.

BACKGROUND: Kasai portoenterostomy (KPE) remains the primary intervention for biliary atresia (BA), but its outcomes are highly variable. Reliable prognostic biomarkers remain elusive, complicating the management and prediction of postoperative progression. METHOD: Liver biopsies from BA patients taken at and after KPE (post-KPE) were used to generate organoids for RNA-sequencing analysis. Control organoids were derived from non-BA livers. Differential gene expression and enrichment analyses were performed to assess post-KPE transcriptomic changes between native liver survivors (NLS) and patients who eventually became liver transplant recipients (LTR). RESULTS: Organoid datasets: 70 from liver biopsies at KPE (10 patients), 112 from post-KPE livers (13 livers; 12 patients), and 47 from control livers (9 patients). At KPE, BA organoids displayed mainly hepatocyte expression, a trait notably reduced in control organoids. Similarly, post-KPE organoids from NLS revealed a significant decrease in hepatocyte expression features and an overall increase in cholangiocyte expression features. A similar hepatocyte-to-cholangiocyte expression transition was evidenced in paired liver organoids (at- and post-KPE) generated from an NLS. In contrast, post-KPE organoids from LTR maintained a high level of hepatocyte expression features. CONCLUSION: Our study demonstrated that an elevated expression of hepatocyte features in KPE organoids may indicate aberrant cholangiocyte development in BA livers. In contrast, a post-KPE hepatocyte-to-cholangiocyte expression transition in NLS may imply effective biliary recovery. The lack of this transition in LTR organoids indicates ongoing disease progression, highlighting the potential for organoid-based transcriptomic profiling to inform KPE success and guide BA management. LEVEL OF EVIDENCE: Level III.

Retrospective analysis of the standardized BARD criteria for acute cholangitis in biliary atresia patients.

Objectives: In 2022, the Biliary Atresia and Related Diseases (BARD) community reached a consensus for the definition of suspected and confirmed cholangitis for biliary atresia (BA) patients after hepatoportoenterostomy (HPE). This study assessed the new standardized BARD definition in a retrospective, multicenter cohort study. Methods: We included BA cases managed between 2010 and 2020 at the Hannover Medical School and Geneva University Hospitals' Swiss Pediatric Liver Center. The standardized BARD cholangitis definition assesses four clinical items and four imaging/laboratory items to define cholangitis. The definition was retrospectively applied to all BA cases having presented, according to their physician, cholangitis within the first year after the HPE. The diagnosis defined by the standardized BARD definition was compared with the final clinical diagnosis made by physicians. The Spearman's correlation coefficient was used to test for correlation between diagnoses made by standardized and clinical appreciation. Results: Of 185 consecutive BA patients, 59 (32%) had at least one episode of cholangitis within the first year after HPE. The correlation between the clinician's impression and the standardized BARD definition was very strong (r = 0.8). Confirmed cholangitis definition coincided with the clinician's impression (2.5 [±0.7]/4 clinical items, 2.6 [±0.5]/4 imaging/laboratory items). For suspected cholangitis, the threshold for diagnosis was lower within the standardized BARD definition (1.1 [±0.3]/4 clinical items, 2.2 [±0.8]/4 laboratory/imaging items). Conclusions: This first retrospective application of the standardized BARD cholangitis definition reveals a very strong correlation with the physician's assessment before standardization. A prospective study is needed to further refine the standardized definition for cholangitis in BA patients.

Biliary Atresia Associated With Jejunal Atresia: A Case Report.

A girl who was born at 40 weeks of gestation weighing 3800 g presented with bilious vomiting and abdominal distension shortly after birth. A lower gastrointestinal contrast study showed a microcolon with small bowel atresia. Subsequently, laparotomy, small bowel resection and anastomosis were done. Intra-operative findings noted jejunal atresia type 3a. Post-operatively, the patient developed persistent conjugated hyperbilirubinaemia and hence, magnetic resonance cholangiopancreatography (MRCP) was performed. MRCP revealed possible biliary atresia (BA) of which the patient underwent Kasai hepato-porto-enterostomy. We reported a rare case of double pathology involving jejunal atresia and BA, describing its aetiology, characteristics and treatment availability based on literature.

Timing of Kasai Procedure for Biliary Atresia: An Analysis of the Pediatric National Surgical Quality Improvement Program Database.

INTRODUCTION: Biliary atresia is a rare liver disease of unknown etiology affecting approximately 1 in 10,000 children. This disease initially presents as inflammatory obstruction of bile ducts leading to cholestasis and eventually fibrosis of hepatic tissue. Affected patients are ideally treated early with portoenterostomy (Kasai procedure) as age at surgery is an important prognostic factor for native liver survival and need for liver transplant. This study aimed to evaluate the age at which patients in the United States are receiving this procedure. METHODS: The American College of Surgeons National Surgical Quality Improvement Program Pediatric database was used to identify patients between 2012 and 2021 who underwent a primary procedure of portoenterostomy. The age at time of surgery and perioperative analysis was performed. The data underwent simple descriptive statistics. RESULTS: Eight hundred twenty four patients were identified who underwent Kasai procedure. Four hundred seventy four (58.2%) were female with the predominant race being White (49.5%). The median age at surgery was 57 d old (interquartile range 41-71). Readmission and reoperation rates within 30 d were 30% and 15.2%, respectively. There were no deaths within 30 d. CONCLUSIONS: Within the National Surgical Quality Improvement Program database, the median age of pediatric patients undergoing Kasai procedure for biliary atresia in the United States exceeds the goal of 45 d. Further studies are needed to investigate factors that may affect time to diagnosis and time to Kasai procedure.

Kasai Portoenterostomy, Successful Liver Transplantation, and Immunosuppressive Therapy for Biliary Atresia in a Female Baby: A Case Report.

Background: Biliary atresia (BA) is a severe neonatal progressive cholangiopathy of unknown etiology. A timely Kasai portoenterostomy (KPE) improves survival of the native liver in patients with BA, although liver transplantation remains the ultimate treatment for most (60%-80%) patients. However, postoperative adverse effects of liver transplantation may be significant. In addition, patients require lifelong immunosuppressive therapy after liver transplantation. Case Summary: Here, we report a case of a newborn female baby (birthday: 10-03-2018) with congenital BA (confirmed at 76 days of life) who survived KPE (first surgery at 85 days of life) and underwent successful living-related liver transplantation (LRLT) (second surgery at 194 days of life). Additionally, we reviewed the existing literature on BA. After KPE (at 85 days of life), the liver function of the baby did not improve, and the indicators of liver and kidney function showed a trend of aggravation, indicating that the liver function had been seriously damaged before KPE (at 85 days of life), demonstrating the urgent need for liver transplantation surgery. The female baby survived after part of her father's liver was successfully transplanted into her body (at 194 days of life). The patient recovered successfully. No other diseases were found at the 4-year follow-up, and all indices of liver and kidney functions tended to be normal. Conclusion: This case highlights the following. Postoperative alkaline phosphatase was consistently above the normal range, although the reason for this was unclear; neither tacrolimus nor cyclosporine A has formulations designed specifically for infants, which does not meet the needs of clinical individualized medication, suggesting that these anti-rejection drugs are future development directions. Only one case of congenital BA has been found thus far in Hefei, and this case has extremely important reference significance for the prevention, treatment, and diagnosis of BA in Hefei, Anhui province.

Inflammation patterns in early post-operative cholangitis predict long-term outcomes in biliary atresia: a potential role of non-suppurative cholangitis.

PURPOSE: Frequent post-operative cholangitis in biliary atresia (BA) affects the long-term native liver survival. This study assessed the characteristics of early cholangitis and their influence on the prognosis. METHODS: Forty-three patients with BA who underwent surgery between 2000 and 2020 were analyzed for routine inflammatory markers. Early cholangitis characteristics were compared between native liver survivor (NLS) and living donor liver transplant (LDLT) patients. RESULTS: Among the 43 patients, 30 (69.8%) experienced 130 episodes of cholangitis. In the area under the receiver operating characteristics curve (AUROC) analysis, the cutoff value of the total cholangitis episodes was 3, with an area under the AUROC curve of 0.695 (95% confidence interval 0.522-0.868). Before 3 years old, 113 episodes (86.9%) of cholangitis were observed. The white blood cell, C-reactive protein, and alanine aminotransferase values at cholangitis onset did not markedly differ between the LDLT and NLS groups. Conversely, the neutrophil-to-lymphocyte ratio in the NLS group was significantly lower than in the LDLT group (0.85 vs. 1.63, p < 0.001). CONCLUSIONS: Cholangitis in the NLS group was lymphocyte-dominant and atypical in its pathogenesis. Lymphocyte-dominant cholangitis is non-suppurative, and future research should clarify its pathogenesis to improve the treatment and prognosis of BA.

Cholestasis after Kasai operation predicts portal hypertension in native liver survivors of biliary atresia: a multicenter study.

PURPOSE: This study evaluated portal hypertension (PHT) and its predictors among native liver survivors (NLS) of biliary atresia (BA) after Kasai portoenterostomy (KPE). METHODS: This was a multicenter study using prospectively collected data. The subjects were patients who remained transplant-free for 5 years after KPE. Their status of PHT was evaluated and variables that predicted PHT were determined by regression analysis and receiver operating characteristic (ROC) curve. RESULTS: Six centers from East Asia participated in this study and 320 subjects with KPE between 1980 to 2018 were analyzed. The mean follow-up period was 10.6 ± 6.2 years. At the 5th year after KPE, PHT was found in 37.8% of the subjects (n = 121). Patients with KPE done before day 41 of life had the lowest percentage of PHT compared to operation at older age. At 12 months after KPE, PHT + ve subjects had a higher bilirubin level (27.1 ± 11.7 vs 12.3 ± 7.9 µmol/L, p = 0.000) and persistent jaundice conferred a higher risk for PHT (OR = 12.9 [9.2-15.4], p = 0.000). ROC analysis demonstrated that a bilirubin level above 38 µmol/L at 12 months after KPE predicted PHT development (sensitivity: 78%, specificity: 60%, AUROC: 0.75). CONCLUSIONS: In BA, early KPE protects against the development of PHT among NLSs. Patients with persistent cholestasis at one year after KPE are at a higher risk of this complication. They should receive a more vigilant follow-up. LEVEL OF EVIDENCE: Level III.

Use of stool color card as screening tool for biliary atresia in resource-constraint country.

Aim: The study was aimed to find out the efficacy of a stool color card (SCC) in differentiating biliary atresia (BA) from non-BA in resource-limited countries. Background: stool color screening system was introduced in 2004 which lead to marked improvement in sensitivity of detecting BA. Methods: This cross-sectional observational study was conducted from January, 2019 through July, 2022 on purposively sampled infants who developed jaundice before three months of age, had direct bilirubin of > 20 % of total with pale stool and dark urine. Results: 144 cases (male, 96) were included in the study and their mean age at admission was 87.3±37.2 days and mean age at onset of jaundice was 6.1±7.7 days. BA was confirmed in 106 (73.6%) cases and 38 (26.4%) children were in non-BA group. Frequency of persistent pale stool between BA and non- BA were 88 vs 8 (83.0 % Vs 21.0 %) which was highly significant (p=0.000). Mean difference of total and direct serum bilirubin, median alanine transferase and alkaline phosphatase were not statistically significant between two groups. Median of serum gamma glutamyl transpeptidase (GGT) in BA was 570 U/L and in non-BA it was 138.0 U/L which was statistically significant (p=0.000). The sensitivity, specificity, positive predictive value, negative predictive value and accuracy of SCC were 83%, 78.9%, 91.7%, 62.5% and 81.9% respectively. Conclusion: SCC has good sensitivity to diagnose BA but failed to prove better specificity to rely simply on it. SCC may be used as early screening tool for prompt referral to appropriate medical care centers for final evaluation of BA.

Delphi Method Analysis and Consensus of Prevalent Distinctive Practices for Biliary Atresia Management after Kasai Portoenterostomy.

Background: Extrahepatic biliary atresia (BA) is seen in infants, with an incidence of 1 in 15,000 live births. The presentation is progressive jaundice, dark-colored urine, and clay-colored stools. Kasai portoenterostomy (KPE) is the commonly performed surgical procedure in these patients. Postoperatively, phenobarbitone, ursodeoxycholic acid (UDCA), steroids, and other drugs are given to improve bile drainage and prevent inflammation and fibrosis. However, a definitive protocol regarding the need for different drugs, dosage, and duration varies across individual surgeons and centers. No universally accepted protocol exists for postoperative management after KPE. Aim: The aim of this study was to know the prevailing postoperative management of BA by subject experts and use the Delphi process to know if the experts want to change their practice based on the results from the survey. Material and Methods: A questionnaire was made after discussing with two experts in the field of BA. The questionnaire was mailed to 25 subject experts. The first survey data were analyzed and shared with all responders. In the second survey, change in the management based on the results from the first survey was assessed. Results: The Delphi questionnaire was answered by 17 experts. Postoperatively, prophylactic antibiotics are prescribed for 6-12 weeks by around 40% and >12 weeks by 30% of respondents. Phenobarbitone is prescribed for <3 months by nearly 50%. UDCA is prescribed for <3 months, ≤6 months, and 6 months-1 year by 47.1%, 23.5%, and 23.5% responders, respectively. Nearly 50% prescribe steroids (mostly prednisolone), and among them, two-thirds prescribe it for 6-12 weeks. Approximately 60% give antiviral drugs to children who are cytomegalovirus immunoglobulin M positive. In our survey, 50% of experts perform 5-10 KPE per year, and 25% each perform 10-15 and >15 KPE per year. The second survey noted that a significant percentage of responders want to change their practice according to consensus. Conclusion: From our Delphi survey, an overview of the postoperative management of BA could be made. However, multicentric studies are required for uniform protocol on the postoperative management of BA.

Porta hepatis lymph nodes on US: not only identify biliary atresia but also predict outcomes after Kasai portoenterostomy surgery.

OBJECTIVES: To evaluate the usefulness of porta hepatis lymph nodes (PHLNs) on ultrasonography (US) scans in diagnosing biliary atresia (BA) and predicting the outcomes after Kasai portoenterostomy (KPE) surgery. METHODS: A total of 668 patients from one hospital were enrolled in the study (542 non-BA and 126 BA). The independent and combined diagnostic efficacy of PHLNs, triangular cord (TC) thickness, and gallbladder morphology were assessed by drawing the receiver operating characteristic (ROC) curves and counting the area under the ROC curve (AUC), sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV). The US features, histopathological findings of PHLNs, and serum total bilirubin (TBIL) levels 3 months post-KPE were correlated. RESULTS: The AUC, sensitivity, specificity, PPV, and NPV of PHLNs with hyperechogenicity and a maximum length larger than 8.4 mm were 0.898, 81.8%, 97.8%, 89.6%, and 95.8%, respectively. The combination of PHLNs, TC thickness, and gallbladder morphology achieved the best overall diagnostic efficacy among all indicators with an AUC of 0.927 and a sensitivity of 99.2%. The germinal center number and bile particle number of PHLNs were positively correlated with pathological size and US echogenicity intensity of PHLNs, respectively (r = 0.591, 0.377, p = 0.001, 0.004). The pathological size of PHLNs in BA patients was negatively correlated with jaundice clearance status 3 months after KPE surgery (r = -0.385, p = 0.047). CONCLUSION: PHLNs with hyperechogenicity and a maximum length > 8.4 mm are useful US indicators for BA diagnosis. Additionally, the enlargement of PHLNs might play a role in predicting outcomes of KPE surgery. CRITICAL RELEVANCE STATEMENT: The article proposed for the first time that PHLNs with hyperechogenicity and a maximum length > 8.4 mm are a useful US indicator for diagnosing BA. KEY POINTS: PHLNs may be helpful in diagnosing BA and predicting outcomes after surgery. Enlarged hyperechoic PHLNs are a useful diagnostic indicator for BA, and play a role in predicting surgical outcomes. These findings can assist clinicians in more accurately diagnosing BA, enabling more timely treatments.

Biliary atresia-splenic malformation (BASM) syndrome: A case report.

INTRODUCTION AND IMPORTANCE: Biliary atresia (BA) is characterized by the presence of persistent cholestatic jaundice during the neonatal period. This group of patients is at higher risk for liver disease and/or portal hypertension compared with other chronic liver diseases. CASE PRESENTATION: We present a newborn patient who had biliary stools early postnatally, but her gallbladder was not seen on radiological examination and was referred to our clinic. On examination, polysplenia was seen, and the inferior vena cava was not seen. The patient's stool color changed to a creamy stool without bile three weeks postnatally. The neonate was diagnosed with biliary atresia and splenic malformation (BASM) syndrome. The patient underwent a Kasai portoenterostomy in the 3rd week of age and was preoperatively diagnosed with malrotation and treated with a Ladd procedure at the same time. The patient had a postoperative stool with bile and decreased bilirubinemia with a normal defecation period. CLINICAL DISCUSSION: BASM syndrome, clinically has different causes and a worse prognosis than isolated BA. The syndrome can lead to end-stage cirrhosis and liver failure if left untreated. Although timely Kasai surgery is the standard treatment for BA, the age of the patient at the time of the surgery is different depending on BA groups. CONCLUSION: The most important prognostic factors for BASM syndrome are early diagnosis and treatment. Despite this, the patients need careful follow-up. Postoperative adjuvant steroid therapy may have a good impact on the outcomes.

Knowledge and practice of health workers towards maternal and child health in the Democratic Republic of the Congo: a cross-sectional study.

BACKGROUND: The burden of maternal and child mortality is high in the Democratic Republic of the Congo (DRC). While health workers (HWs) with adequate knowledge and practice of maternal and child health (MCH) are crucial to reduce this burden, the skill level of HWs in charge of MCH in the DRC is currently insufficient. This study aimed to assess the knowledge and practice of HWs towards MCH in Kasai and Maniema, two DRC provinces with very high maternal mortality ratios and under-5 mortality rates. METHODS: This cross-sectional study was conducted in 96 health facilities of Kasai and Maniema provinces in 2019. All HWs in charge of MCH were eligible for the study. Data were collected using a structured questionnaire containing 76 questions on knowledge and practice of MCH. Analyses were performed using the Wilcoxon-Mann-Whitney test, Kendall's correlation test, and a multivariate linear mixed regression model. RESULTS: Among participating HWs, 42.6% were A2 nurses (lowest qualification), 81.9% had no up-to-date training in MCH, and 48.4% had only 1-5 years of experience in MCH. In the two provinces combined, about half of HWs had poor knowledge (50.6%) and poor practice (53.3%) of MCH. Knowledge and practice scores were higher in Maniema than in Kasai (P < 0.001). Good knowledge and practice scores were significantly associated with high qualification (P = 0.001), continuing up-to-date training in MCH (P = 0.009), and 6 years of experience or more in MCH (P = 0.01). CONCLUSION: In Maniema and Kasai provinces, about half of HWs had poor knowledge and poor practice of MCH. The conversion of A1 nurses into midwives as well as the provision of up-to-date training in MCH, supervision, and mentorship could improve the skill level of HWs and could thus reduce the burden of MCH in the DRC.

This study assessed the knowledge and practice of health workers (HWs) towards maternal and child health (MCH) in Kasai and Maniema, two provinces of the Democratic Republic of the Congo (DRC) with very high maternal and child mortality rates. About half of surveyed HWs had poor knowledge and poor practice of MCH. Good knowledge and good practice were associated with high qualification, up-to-date training, and 6 years of experience or more in MCH. The conversion of A1 nurses into midwives as well as the provision of up-to-date training in MCH, supervision, and mentorship could improve the skill level of HWs and could thus reduce the burden of MCH in the DRC.

Primary Liver Transplant in Biliary Atresia: The Case for and Against.

The role of liver transplantation as a primary procedure in biliary atresia has been argued over for at least 40 years, indeed since the coming of age of safe liver transplantation during the 1980s. Yet, it is not a common option in most series (usually ≤5%) and typically reserved for those with late presentations (arguably >100 days) with established cirrhosis. This review presents the pros and cons of primary liver transplant. The pros are based upon the observation that at best a Kasai portoenterostomy (KPE) is simply palliative in most, and at worse has no effect whatsoever on restoration of bile flow and is therefore pointless. Set against this are the cons: there is a dearth of prognostic tests (clinical, biochemical, or histological) at the time of presentation which may predict inevitable failure; the possibility of long-term native liver survival to adulthood in a proportion (albeit a minority); and the implied increased need for donor organs suitable for infants - a stressor for an already overstressed system. Improving results from KPE in terms of increasing the proportions clearing their jaundice and minimizing the effects of chronic liver fibrosis and cirrhosis would surely limit the siren calls for primary transplants but the key must be better discrimination at presentation with the use of biomarkers (circulatory or histological, individually or together) to enable better decision making.

"Where Did This Come From?": Antibiotic Prophylaxis in Biliary Atresia After Kasai Procedure.

For patients with Biliary atresia, antibiotic prophylaxis after Kasai portoenterostomy is a common practice. Societal guidelines often cite one reference as supportive evidence for this practice. In this paper, we go back to review the quality of this evidence and suggest more research is required to demonstrate the efficacy of antibiotic prophylaxis in this population.

Biliary atresia in preterm infants: a single center experience and review of literature.

Introduction: The diagnosis of biliary atresia (BA) remains challenging, and there is still uncertainty regarding the optimal time to perform a Kasai portoenterostomy (KPE). Little is known about the difficulties in the diagnosis and outcomes of BA in preterm infants (PBA). This study, which represents the first Italian report of preterm infants with BA, aims to describe a single-center experience of BA in preterm newborns. Methods: We retrospectively reviewed all infants consecutively diagnosed with BA who underwent a Kasai procedure at the Bambino Gesù Children's Hospital between January 1998 and December 2021. Prematurity was defined as a gestational age (GA) of <37 weeks. Demographic, laboratory, and histology data were recorded, and the main outcomes considered were clearance of jaundice (COJ), native liver survival, and mortality. Results: A total of 21 PBA were compared with 117 term BA controls (TBA). The median GA of PBA was 35.1 (32-36.1) weeks, with a mean birth weight of 2,100 (1,897-2,800) g. Age at first presentation was significantly lower in PBA patients: 46 (22-68) vs. 61 (44-72) days; p = 0.02. The median age at KPE was similar between the two groups: 70 days (33 corrected) for PBA vs. 67 in TBA; p = 0.8. At the time of surgery, median serum bilirubin was lower in the PBA group (7.7 vs. 8.6 mg/dl, p = 0.04). Similarly, the median APRi at the time of KPE was lower but not significant in the PBA group: 1.09 vs. 1.16; p = 0.8. No differences were found in terms of COJ between the PBA and TBA groups: n = 9 (43%) vs. 34 (35%); p = 0.2. Overall native liver survival was similar between the two groups: 8.6 (4.8-12.2) for the PBA group vs. 7.6 (5.6-9.5) years for the TBA group with no significant differences; p = 0.45. Post-KPE native liver survival was similar between the two groups: 38% vs. 52% at 5 years for the TBA and PBA groups, respectively; p = 0.54. Conclusion: The PBA and TBA groups appear to have similar outcomes in terms of COJ, overall native liver survival, and 5-year liver survival. Considering the corrected GA, early KPE is related to lower cholestatic damage. Further multicenter studies are required.